Endoscopic resection of intracranial dermoid cysts.

INTRODUCTION: Nasal dermoid cysts are congenital lesions which are often diagnosed in infancy or childhood. However, a small number present in adulthood, and some extend intracranially. Traditional treatment for the intracranial portion of these cysts includes frontal craniotomy. CASE REPORTS: Two intracranial dermoid cysts were resected via a transnasal endoscopic approach, using 70° nasal endoscopy for complete visualisation and intracranial tumour removal. We describe our technique for the procedure itself and for reconstruction of the skull base defect. DISCUSSION AND CONCLUSION: The endoscopic transnasal skull base approach is an excellent alternative to a traditional frontal craniotomy, to achieve complete resection of intracranial dermoid cysts.

Imaging manifestations of progressive multifocal leukoencephalopathy.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by reactivation of JC virus in immunosuppressed patients. The diagnosis is usually suggested on imaging and confirmed by cerebrospinal fluid polymerase chain reaction (PCR) for JC virus DNA. In this article, we review the imaging manifestations of PML on computed tomography (CT), magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI), diffusion tensor imaging (DTI), MR spectroscopy, single photon-emission computed tomography (SPECT) and positron-emission tomography (PET), and outline the role of imaging in follow-up and prognostication.

JC virus infection of the brain.

Since its initial description, there have been significant changes in the epidemiology, pathogenesis, and clinical and imaging manifestations of JCV infection of brain. The most common clinical manifestation is PML. Other recently described CNS manifestations are JCE, JCVGCN, and JCM. Although AIDS is the most common predisposing factor for JCV reactivation, there is increasing incidence of brain manifestations of JCV reactivation in non-HIV settings, including different rheumatologic, hematologic, and oncologic conditions; monoclonal antibody therapy; transplant recipients; primary immunodeficiency syndromes; and even in patients without any recognizable immune deficiency. IRIS may develop secondary to restoration of immunity in HIV-positive patients with PML receiving antiretroviral therapy. This is of profound clinical significance and needs to be diagnosed promptly. Imaging plays a crucial role in the diagnosis of the disease, monitoring of treatment response, identifying disease progression, and predicting prognosis. In this article, current understanding of the epidemiology, pathogenesis, clinical presentations, and all aspects of imaging of JCV infection of the brain have been comprehensively reviewed.

Correlation of MR imaging findings and clinical manifestations in neurosarcoidosis.

BACKGROUND AND PURPOSE: MR imaging is widely used for the diagnosis and follow-up of neurosarcoidosis lesions. However, the temporal evolution of imaging abnormalities and the prognostic significance of imaging features is not well understood. We undertook a retrospective study of patients with biopsy-proved or clinically diagnosed neurosarcoidosis for the following reasons: 1) to assess concordance between abnormalities noted on MR imaging with neurologic symptoms at presentation; 2) to correlate changes in imaging findings during follow-up with clinical improvement or worsening; and 3) to identify imaging features that may have prognostic significance. MATERIALS AND METHODS: We reviewed radiologic records from 1999 to 2008 of patients with biopsy-proved or clinically diagnosed neurosarcoidosis and correlated MR imaging findings in these patients with their demographic characteristics, clinical features, and symptomatic responses during follow-up. RESULTS: Thirty-two patients were included in the study. Cranial nerve lesions and leptomeningeal disease identified on MR imaging were symptomatic in 64% patients, dural lesions were associated with symptoms in 28% patients, but nonenhancing white matter lesions did not have correlating symptoms. During follow-up, MR imaging findings generally changed in concordance with the change in clinical symptoms (80% patients). Cranial nerve enhancement (9/11) and spinal lesions (5/8) were most likely to resolve with clinical improvement, whereas dural lesions (6/11), enhancing parenchymal lesions (3/7), and non-enhancing parenchymal lesions (0/4) were less likely to change during therapy. Patients with > or =1 enhancing T2-hypointense lesion (4/7, 57%) were less likely to show clinical improvement than those without such lesions (12/13, 92%). CONCLUSIONS: Although many sarcoid-related MR imaging abnormalities were not associated with correlating symptoms at presentation, there was a high degree of concordance between changes in clinical symptoms and MR imaging abnormalities during follow-up. Enhancing T2-hypointense dural and parenchymal lesions were associated with suboptimal imaging and clinical response to immunosuppressive therapy.

Imaging and clinical characteristics of temporal bone meningioma.

BACKGROUND AND PURPOSE: Imaging characteristics of temporal bone meningioma have not been previously reported in the literature. CT and MR imaging findings in 13 cases of temporal bone meningioma are reviewed to define specific imaging features. METHODS: A retrospective review of our institutional case archive revealed 13 cases of histologically confirmed temporal bone meningioma. CT and MR imaging studies were reviewed to characterize mass location, vector of spread, bone changes, enhancement characteristics, and intracranial patterns of involvement. Clinical presenting signs and symptoms were correlated with imaging findings. RESULTS: Thirteen temporal bone meningiomas were reviewed in 8 women and 5 men, aged 18-65 years. Meningiomas were stratified into 3 groups on the basis of location and tumor vector of spread. There were 6 tegmen tympani, 5 jugular foramen (JF), and 2 internal auditory canal (IAC) meningiomas. Tegmen tympani and JF meningiomas were characterized by spread to the middle ear cavity. IAC meningiomas, by contrast, spread to the cochlea and vestibule. Hearing loss was the most common clinical presenting feature in all cases of temporal bone meningioma (10/13). The presence of tumor adjacent to the ossicles strongly correlated with conductive hearing loss (7/9). CONCLUSION: Meningioma involving the temporal bone is rare. Three subgroups of meningioma exist in this location: tegmen tympani, JF, and IAC meningioma. Tegmen tympani and JF meningiomas spread to the middle ear cavity. IAC meningiomas spread to intralabyrinthine structures. Conductive hearing loss is commonly seen in these patients and can be surgically correctable.

High-dose chemotherapy with autologous haemopoietic support for advanced ovarian cancer in first complete remission: retrospective analysis from the Solid Tumour Registry of the European Group for Blood and Marrow Transplantation (EBMT).

The majority of advanced ovarian cancer patients achieve an objective response following chemotherapy; however, only 20-30% are in remission after 5 years. Intraperitoneal or high-dose chemotherapy (HDC) may prolong disease-free and overall survival (OS) in patients with platinum-sensitive, small volume disease. To better define the subsets of patients who might benefit from HDC, we performed a retrospective analysis on 91 patients in 1st complete remission (CR) treated from 21 centres of the EBMT group. At a median follow-up of 48 months, median time-to-progression (TTP) and OS were 21.2 and 44.4 months, respectively. Tumour grade, stage, residual disease, disease status before HDC, type and year of transplant, source of haemopoietic progenitors and use of haemopoietic growth factors (HGF) after transplant were analysed for TTP and OS. The only significant parameter was the use of HGF: median OS for patients receiving or not receiving HGF was 46.2 vs 17.8 months, respectively (P: 0.035); this difference was maintained after multivariate analysis (P: 0.02). Our analysis does not identify any subgroup of patients in 1st CR who can benefit from HDC; however, median survival of patient with no residual disease has not been reached. The role of HGF after HDC deserves further investigation.

Imaging of vascular lesions of the head and neck.

The imaging armamentarium for imaging head and neck vascular lesions and the imaging features of each have been reviewed. Imaging is an indispensable part of the diagnosis and treatment planning of these lesions. The architecture and vascularity of these lesions are keys to their correct diagnosis. High-flow lesions (AVMs and hemangiomas) can be distinguished readily from low-flow lesions (venous malformations and lymphatic malformations) with these techniques, without the need for conventional angiography in the majority of cases. More-over, the architecture of the lesions depicted on imaging studies can lead to a reasonably specific diagnosis. MR imaging is the best tool for this assessment, but complementary information from ultrasound or CT can help arrive at the correct diagnosis when the results of MR imaging are equivocal. Ultrasound can correctly characterize the lesion as high flow or low flow but is limited in its ability to determine the full extent of the lesion. The usefulness of CT is more limited. Application of newer CT techniques (multidetector helical CT with image reconstruction) may increase the role of CT in high-flow lesions, but the greater soft tissue contrast sensitivity of MR imaging remains its strong suit. Conventional angiography is usually reserved for pretherapeutic evaluation.

Cranial MR imaging of osteopetrosis.

BACKGROUND AND PURPOSE: The purpose of this study was to describe the cranial MR imaging manifestations of osteopetrosis. These features have not previously been reported in the literature. METHODS: Cranial MR studies, obtained with a uniform imaging protocol, were reviewed in 47 patients with osteopetrosis. Thirty-four patients had autosomal recessive (malignant) osteopetrosis (AROP), seven had intermediate osteopetrosis (IOP), and six had either type I or type II autosomal dominant osteopetrosis (ADOP I or II). The prevalence of abnormalities was tabulated and compared with the specific osteopetrosis variants. RESULTS: All patients with osteopetrosis had thickening and sclerosis of the calvaria. Ventriculomegaly, tonsillar herniation, proptosis, and dural venous sinus stenosis were observed in the majority of patients with AROP and ADOP I. Optic nerve sheath dilatation occurred in many of the patients with AROP and in all patients with ADOP I. Acquired cephaloceles were also observed only in these two groups. Optic nerve atrophy and optic canal stenosis were observed in a majority of patients with AROP, IOP, and ADOP II. Middle ear fluid was prevalent in AROP and IOP, present in over half the patients in each group. Features seen most prevalently, or exclusively, in AROP included stenosis of the internal carotid and vertebral arteries and extramedullary hematopoiesis. CONCLUSION: The cranial MR imaging features of osteopetrosis are both shared and unique among the various subtypes of the disease. The specific cranial and intracranial manifestations reflect the predominant calvarial or skull base patterns of bone thickening. The unique features seen in patients with AROP probably reflect the early age of onset and the greater severity of this form of the disease.

Colangiopancreatografía retrógrada endoscópica / Endoscopic retrograde cholangiopancreatography

Se presenta un estudio analitico prospectivo de la colangiopancreatografia retrograda endoscopica (CPRE), realizada a 112 pacientes, entre julio de 1998 y marzo de 2000, cuya principal indicacion fue el diagnostico de la patologia del confluente bilio-pancreático. En el se resume nuestra experiencia personal.

Untethered filum terminale presenting as cauda equina lesion.

We report the case of a young girl with recurrent bladder dysfunction. Magnetic resonance imaging performed for evaluation of initial urologic symptoms revealed a low-lying conus medullaris. She underwent an L5 laminectomy and cord untethering by sectioning of the filum terminale. After initial improvement of bladder function, her symptoms returned 4 years later. Repeat magnetic resonance imaging demonstrated a new intradural lesion at L2. At surgery she was found to have an untethered, thickened, coiled filum terminale at L2.

Symptomatic spinal calcinosis in systemic sclerosis (scleroderma).

Two patients with diffuse cutaneous systemic sclerosis and spinal calcification, involving the lumbar spine in one and the cervical spine in the other, are described. Computed tomography-guided aspiration of the calcific masses was performed, and material aspirated from one patient was shown to be apatite, Ca5(PO4)3OH. One patient showed improvement following lumbar laminotomy, hemilaminectomy, and diskectomy.

Cystlike white matter lesions in tuberous sclerosis.

PURPOSE: To investigate the presence of small cystlike structures in the cerebral hemispheric white matter on MR images of patients with tuberous sclerosis. METHODS: The MR images of 18 consecutive patients with tuberous sclerosis were reviewed retrospectively. RESULTS: Eight of the 18 patients were found to have cystlike structures in the cerebral white matter. The signal intensity of these lesions was isointense with cerebrospinal fluid on T1-, proton density-, and T2-weighted images. Four patients were imaged with a fluid-attenuated inversion recovery sequence, which in each case also showed fluid-type signal in these areas. Three of the patients had CT for correlation, and these scans supported the diagnosis of cystic lesions. Cysts ranged in number from one to 12 per patient and were usually smaller than 1 cm. The most common location was adjacent to the occipital horn or trigone of the lateral ventricle (six of eight patients). Less frequent sites were near the frontal horns, in the corpus callosum, and in the deep white matter near the body of the lateral ventricle. Cysts in five patients were either immediately adjacent to a cortical tuber or in the center of a white matter dysplastic lesion. A cyst in one patient had septa, and none of the cysts enhanced. CONCLUSIONS: Cystlike structures in the cerebral hemispheric white matter were seen on the MR images of 44% of 18 patients with tuberous sclerosis. Whether these findings represent cystic degeneration of dysplastic tissue or are unrelated to the disease process of tuberous sclerosis is unknown. More than one pathogenesis may exist.

Developmental neural abnormalities and seizures in epidermal nevus syndrome.

The epidermal nevus syndrome (ENS) is an unusual neurocutaneous disorder consisting of the combination of an epidermal nevus and a central nervous system (CNS), ophthalmological, and/or skeletal abnormality. The study reports four new patients with ENS. Each had a confirmatory biopsy of the epidermal nevus, abnormal neurological examination findings, and documented CNS anatomical studies by imaging or autopsy. The paper also reviews the literature in English to determine neurological abnormalities found in skin-biopsy-proven cases of ENS. Hemi-atrophy, hemimegalencephaly, migrational abnormalities and vascular anomalies were found to be the most frequent intracranial abnormalities associated with ENS. Seizures and/or disabling moderate to severe developmental delays were present in a majority of patients. Seizure onset during the neonatal period or early infancy was associated with major hemispheric malformations. Neuroectodermal-derived ocular lesions were often bilateral. No consistent relation between laterality of the nevus and laterality of CNS abnormalities was found, supporting the gene mosaicism theory of pathogenesis.

Petrous carotid canal stenosis in malignant osteopetrosis: CT documentation with MR angiographic correlation.

PURPOSE: To study the association between petrous carotid canal (PCC) and internal carotid artery (ICA) stenoses in patients with malignant osteopetrosis. MATERIALS AND METHODS: Mean and minimum PCC diameters obtained from cranial computed tomographic (CT) scans in 20 patients were compared with similar measurements in 52 control subjects. ICA caliber, evaluated with magnetic resonance (MR) arteriography, was correlated with age and PCC dimensions. RESULTS: There was a statistically significant difference between patient and control PCC diameters. There was a strong positive correlation between age and PCC diameter in the control subjects, but only a weakly positive correlation in the patients. One or both ICAs were stenotic on all patient MR arteriograms. MR angiographic stenosis grade correlated positively with age but not with PCC diameters. CONCLUSION: PCC and ICA stenoses occur frequently in patients with malignant osteopetrosis. Bony overgrowth or a "persistent fetal state" may produce the PPC stenoses. The findings do not support progressive PCC narrowing in these patients.

MR of nasolabial cysts.

We report the MR imaging findings in two cases of nasolabial cysts. Demonstration of their extraosseous location with cross-sectional imaging should prevent confusion with maxillary cysts and obviate unwarranted dental or maxillary surgery.

Neurologic complications following bone marrow transplantation for sickle cell disease.

A boy with sickle cell anemia underwent bone marrow transplantation (BMT). He was normal on neurological examination, but had radiologic evidence of an old left frontal lobe infarct, multiple cerebral vascular stenoses and moyamoya collaterals. After BMT he developed seizures with extension of the infarct and subarachnoid hemorrhage. One year later angiography revealed worsening stenosis of the M1 segments of both middle cerebral arteries. At that time an increase in von Willebrand's factor with decreased large molecular weight multimers (LvWF) was observed. We speculate that LvWF dependent, shear-induced platelet aggregation, together with endothelial damage may have contributed to the development of neurologic complications in this patient.